Membranous Nephropathy Flashcards
Definition
Most common cause for nephrotic syndrome
Deposition of IgG and components in the glomerular capillary Wall followed Boy synthesis of new basemant membrane around them
Rarely inflammatory response in the glomeruli of interstitium
Primary MN
Kidney-Limited auto-immune disease and accounts for 80 % of cases
Immune complex mediated against podocyte surface antigen
M-Type phospholipase-A2-receptor 1 (PLA2R) 70-80%
Thrombospondin type 1 Domain-containing 7a (THSD7A) 2-5%
Neural epidermal grwoth factor like Protein 1 (NELL-1) 5-10%
Secondary MN
In 20% of patients
Neoplasm: Carcinomas, especially solid organ (tumors of the lung, colon, breast, and kidney), leukemia, and non-Hodgkin lymphoma
Infections:Malaria, hepatitis B and C, secondary or congenital syphilis, leprosy
Drugs: Penicillamine, gold
Immunologic : Systemic lupus erythematosus, mixed connective tissue disease, thyroiditis, dermatitis herpetiformis, sarcoidosis
Post kidney transplant: Recurrent disease, de novo membranous nephropathy
Miscellaneous: Sickle cell anemia, bovine serum albumin in children
Genes
Neutral Endopeptidase
Bovine Serum Albumin
M-type phospholipase a2 receptor
Thrombospondin Type 1 Domain containing 7a
(THSD7A)
Neural epidermal grwoth factor like protein 1
(NELL-1)
Semaphorin 3B
Exostosin 1&2
Diagnosis
Kidney biopsy
Anti-PLA2R antibodies and anti-THSD7A antibody
Treatment non-specific
Sofien restriction
RR reduction
ACE,ARBs inhibitors
Treatment of Secondary effects (nephrotic syndrome:elevated cholesterol, triglycerides,LDL; normal to low HDL) —> statine
Immuno therapy
Rituximab, or cytoxic+ corticosteroids, or cyclosporine